The Milroy Disease

The Milroy disease is a rare disease that affects the normal function
of the lymphatic system. The lymphatic system produces and transports
fluids and immune cells throughout the body. The transportation
disorders and the accumulation of lymphatic liquid are causing the
lymphatic edema (or lymphedema). Individuals with the Milroy disease
usually develop swelling in the legs shortly after birth. They typically
appear on both halves of the body and are not advancing with time.

Besides the lymphatic edema, Milroy’s disease patients may be born
with an accumulation of fluid in the scrotum (hydrocele) or
malformations of the urethra (the tube that carries the urine from the
bladder to the urethra, outside). Other symptoms may be prominent veins
in the legs, feet fingers in an upward position, verruciform
excrescences. Some patients may develop a non-contagious skin infection
called cellulitis, which deteriorates the lymphatic vessels. This type
of cellulite is more likely in men than in women and it intensify the
edemation process in legs.

Etiology

The Milroy disease develops due to a defect in the development of the
lymphatic vessels in the uterus. At the cellular level, the Milroy
disease is associated with a gene mutation FLT4, which serves to encode
the information to produce a protein called “receiver three of the
vascular endothelial growth factor” (VEGFR-3). This protein regulates
and supports the development of the lymphatic system. The FLT4 gene
mutations affect the growth, the development and the survival of the
lymphoid cells. Given that lymphatic vessels are small or absent, the
lymph is not properly transported and it is stored in the body tissues
forming the lymphatic edema. It is unknown how this mutation is
conditioning the other pathological manifestations of the disease.

Not all individuals with Milroy disease show a FTL-4 gene mutation. In their case, the disease etiology is unclear.

Clinical Manifestations

– The edema occurring at birth, are firm to the touch, and it the skin temperature that it covers is high. The feeling of wood can occur in the area, showing progressive tissue fibrosis.

-Usually, the right foot inferior extremity is involved. The edema usually appears on the front of the dorsal foot and does not extend beyond the knee.

– Patients may show cellulitis, papillomatosis and large-caliber veins in the legs.

– In males, the hydrocele is the second most frequently sign (after edema)

– Usually, one or more family members have congenital lymphatic edema.

-Sometimes the xantoame veruciforme bleeding can occur behind toes.

Diagnosing The Milroy Disease

The basic clues that suggest the diagnosis of the Milroy’s disease
are the edemas and the relevant family history (similar cases in the
family). Currently the 100% accurate diagnosis is done with an
investigation called lymphoscintigraphy, which consists of injecting a
radioactive substance in the leg and a computer monitors its
distribution tracking to identify the exact location of the lymphatic
blockage.

How To Treat

The type of treatment most often practiced in the Milroy disease is
the decongestant treatment. There is no specific treatment but the
disease can be kept under control if it is diagnosed early and it is
treated properly to prevent the complications: infection, pain, skin
problems and in rare cases lymphangiosarcoma, vertebral anomalies,
cerebrovascular malformations.

Prognosis

The long-term prognosis is good, provided it is diagnosed in time and an appropriate treatment is started immediately.

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